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Cushing's symphalangism

Overview

Cushing\'s symphalangism: A rare syndrome characterized by deafness and the fusion of joints in the middle of fingers and toes as well as fusion of hand and foot bones.

Symptoms - Cushing's symphalangism

The list of signs and symptoms mentioned in various sources for Cushing\'s symphalangism includes the 20 symptoms listed below: * Symphalangism * Fusion of carpal bones * Fusion of tarsal bones * Conductive deafness * Crossed eyes * Inability to close hand properly * Permanent flexion of some fingers * Fused foot bones * Fused hand bones * Fused finger joints * Fused toe joints * Short fingers * Curved fingers * Deafness * Dislocated elbow * Clubfoot * Webbed fingers * Weak eye muscles * Fused upper arm bone and radius * Wrist anomalies

Causes - Cushing's symphalangism

In approximately 70% of patients, Cushing’s syndrome results from excessive production of corticotropin and consequent hyperplasia of the adrenal cortex. Overproduction of corticotropin may stem from pituitary hypersecretion (Cushing’s disease), a corticotropin-producing tumor in another organ (particularly bronchogenic or pancreatic cancer), or excessive administration of exogenous glucocorticoids. In the remaining 30% of patients, Cushing’s syndrome results from a cortisol-secreting adrenal tumor, which is usually benign. In infants, the usual cause of Cushing’s syndrome is adrenal carcinoma. Cushing’s syndrome affects 13 of every 1 million people. It’s more common in females than in males and occurs primarily between ages between ages 25 and 40.

Prevention - Cushing's symphalangism

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Diagnosis - Cushing's symphalangism

The phrase \"signs of Cushing\'s symphalangism\" should, strictly speaking, refer only to those signs and symptoms of Cushing\'s symphalangism that are not readily apparent to the patient. The word \"symptoms of Cushing\'s symphalangism\" is the more general meaning; see symptoms of Cushing\'s symphalangism. The signs and symptom information on this page attempts to provide a list of some possible signs and symptoms of Cushing\'s symphalangism. This medical information about signs and symptoms for Cushing\'s symphalangism has been gathered from various sources, may not be fully accurate, and may not be the full list of Cushing\'s symphalangism signs or Cushing\'s symphalangism symptoms. Furthermore, signs and symptoms of Cushing\'s symphalangism may vary on an individual basis for each patient. Only your doctor can provide adequate diagnosis of any signs or symptoms and whether they are indeed Cushing\'s symphalangism symptoms.

Prognosis - Cushing's symphalangism

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Treatment - Cushing's symphalangism

Treatment to restore hormone balance and reverse Cushing’s syndrome may necessitate radiation, drug therapy, or surgery. For example, pituitary-dependent Cushing’s syndrome with adrenal hyperplasia and severe cushingoid symptoms (such as psychosis, poorly controlled diabetes mellitus, osteoporosis, and severe pathologic fractures) may require partial or complete hypophysectomy or pituitary irradiation. If the patient fails to respond, bilateral adrenalectomy may be performed. Nonendocrine corticotropin-producing tumors require excision of the tumor, followed by drug therapy (for example, with mitotane, metyrapone, or aminoglutethimide) to decrease cortisol levels if symptoms persist. Aminoglutethimide and ketoconazole decrease cortisol levels and have been beneficial for many cushingoid patients. Aminoglutethimide alone, or in combination with metyrapone, may also be useful in metastatic adrenal carcinoma. Before surgery, the patient with cushingoid symptoms should have special management to control hypertension, edema, diabetes, and cardiovascular manifestations and to prevent infection. Glucocorticoid administration on the morning of surgery can help prevent acute adrenal hypofunction during surgery. Cortisol therapy is essential during and after surgery, to help the patient tolerate the physiologic stress imposed by removal of the pituitary or adrenals. If normal cortisol production resumes, steroid therapy may be gradually tapered and eventually discontinued. However, bilateral adrenalectomy or total hypophysectomy mandates lifelong steroid replacement therapy to correct hormonal deficiencies.

Resources - Cushing's symphalangism

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